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Cardiomyopathie hypertrophique 肥厚性心肌病
Pub Date : 2005-05-01 DOI: 10.1016/j.emcaa.2005.01.003
P. Charron (Maître de conférences des Universités, praticien hospitalier), M. Komajda (Professeur des Universités, praticien hospitalier)

Hypertrophic cardiomyopathy is characterized by an asymmetric hypertrophy of the left ventricle, especially in the interventricular septum. An outflow gradient in the left ventricle is present in twenty-five percent of cases. The disease is usually hereditary and genes responsible for the disease encode for the sarcomere proteins. The natural course is generally favourable but sometimes complications may occur, such as sudden death or congestive heart failure. Treatment remains difficult. If symptoms are not controlled by beta-blockers or verapamil, and if a pressure gradient is present, surgery with myotomy-myectomy may be proposed. Other treatments in such case are dual-chamber pacing or non-surgical septum reduction (alcohol injection). In patients with high-risk of sudden death, amiodarone or implantable cardioverter-defibrillator can be proposed.

肥厚型心肌病的特点是左心室不对称肥大,尤其是室间隔。25%的病例出现左心室流出梯度。这种疾病通常是遗传性的,导致这种疾病的基因编码肌节蛋白。自然病程通常是有利的,但有时可能会出现并发症,如猝死或充血性心力衰竭。治疗仍然很困难。如果β受体阻滞剂或维拉帕米不能控制症状,并且存在压力梯度,则可以建议进行肌切开髓鞘切除术。这种情况下的其他治疗方法是双腔起搏或非手术隔膜复位(酒精注射)。对于猝死高危患者,可以建议使用胺碘酮或植入式心律转复除颤器。
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引用次数: 0
Hémorrhéologie et cardiologie. Concept, physiopathologie, applications aux affections cardiologiques 血液学和心脏病学。概念、病理生理学、心脏病应用
Pub Date : 2005-05-01 DOI: 10.1016/j.emcaa.2004.12.001
M.-R. Boisseau (Professeur)

The discovery that the blood and the vessel wall constitute a single organ, i.e., that the content definitely influences the container - which is the main object of the haemorrheology - is attributable to A.L. Copley (1910-1992). Due to driving forces, blood flow organizes itself as concentric layers (laminar flow) shearing over each other. Such a shear stress is stronger at the wall level than in the middle of the vessel, where layers are less distinct. Red cells deform and take the shape of layers, allowing blood to become fluid (low viscosity). In vascular areas with decreased pressure, thus lower shear stress, blood becomes more viscous due to the presence of huge red cell aggregates, in relation to fibrinogen level (thyxotropy, red cell aggregation), changing and decreasing the shearing at the wall level. Such a two-phase behaviour of the shear stress characterizes the blood flow reactivity toward the wall. As viscosity measurement consists of plasma and blood viscosity, time and threshold of erythrocyte aggregation appear more accurate determinants. The shear stress mechanotransduction involves endothelial membrane receptors (caveoli, ion channels, integrins), then MAP-kinases systems, and finally transcription factors able to bind specific areas in gene promoters. Over 10 000 shear-sensitive genes have been identified to date. In arteries the shear stress induces NO, which is vasoactive and inhibits platelets. The shearing power at the wall level or at branching zones is often poor, due to pulsations and picks, where subsequently NO production is low and shear-down-regulated functions can occur, mainly leukocyte adhesion and migration. In veins changes in shear are more frequent, particularly in valvulae. Microcirculation is implemented as functional units, exhibiting a vasoactive precapillary side, capillaries with diameters lower than those of red cells and a post-capillary venous side with low output and much decreased shear stress. Endothelial cells are here very active for leukocyte adhesion, inflammation and haemostasis as well. Atherosclerosis stems from zones where monocytes-macrophages are able to enter the wall, bearing large amounts of lipoproteins, the main plaque constituent. As risk factors increase fibrinogen, high red cell aggregates change the wall shear stress and then LDL-receptors can be activated allowing plaques to grow up and extend along the arterial trunks. The atherosclerosis plaque is submitted to high shear upwardly, that activates platelets, but exhibits a stagnant zone downward, where leucocytes adhere and migrate. Myocardial infarct is due to the rupture of a coronary plaque, but its size is related to the rheological factors (fibrinogen, viscosity). Occlusive arterial disease largely exhibits such rheological disorders, with subsequent actions on ischemia. Hemodilution is proposed during acute events, mainly in stroke. Flow restoration (bypass), prevention of risk factors, venous contention and venoa

a.L.Copley(1910-1992)发现血液和血管壁构成了一个单一的器官,即内容物肯定会影响容器——容器是血液流变学的主要对象。由于驱动力的作用,血流组织成相互剪切的同心层(层流)。这种剪应力在壁面比在容器中间更强,在容器中间,层不太明显。红细胞变形并呈层状,使血液变成液体(低粘度)。在压力降低的血管区域,因此剪切应力较低,由于存在与纤维蛋白原水平相关的巨大红细胞聚集体(胸腺缺陷性,红细胞聚集),血液变得更粘稠,从而改变和减少了壁水平的剪切。剪切应力的这种两相行为表征了朝向壁的血流反应性。由于粘度测量由血浆和血液粘度组成,红细胞聚集的时间和阈值似乎是更准确的决定因素。剪切应力机械转导涉及内皮膜受体(小窝、离子通道、整合素),然后是MAP激酶系统,最后是能够结合基因启动子中特定区域的转录因子。到目前为止,已经鉴定出超过10000个剪切敏感基因。在动脉中,剪切应力诱导NO,NO具有血管活性并抑制血小板。壁水平或分支区的剪切力通常较差,这是由于脉动和拾取,随后NO产生较低,并且可能发生剪切下调功能,主要是白细胞粘附和迁移。静脉中剪切力的变化更为频繁,尤其是瓣膜。微循环是作为功能单元实现的,表现出血管活性的毛细血管前侧、直径低于红细胞的毛细血管和具有低输出和大大降低的剪切应力的毛细血管后静脉侧。内皮细胞在白细胞粘附、炎症和止血方面也非常活跃。动脉粥样硬化源于单核细胞-巨噬细胞能够进入壁的区域,携带大量脂蛋白,这是斑块的主要成分。随着危险因素增加纤维蛋白原,高红细胞聚集物改变了壁剪切应力,然后LDL受体可以被激活,使斑块生长并沿着动脉干延伸。动脉粥样硬化斑块向上受到高剪切,激活血小板,但向下呈现停滞区,白细胞在此处粘附和迁移。心肌梗死是由于冠状动脉斑块破裂引起的,但其大小与流变因素(纤维蛋白原、粘度)有关。闭塞性动脉疾病在很大程度上表现出这种流变性疾病,随后对缺血起作用。血液稀释是在急性事件中提出的,主要是在中风中。血流恢复(旁路)、预防危险因素、静脉争用和静脉活性药物可减少血管疾病中流变性疾病的影响。
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引用次数: 0
Troubles cardiovasculaires d'origine thyroïdienne 甲状腺源性心血管疾病
Pub Date : 2005-05-01 DOI: 10.1016/j.emcaa.2005.01.001
Y. Lorcy (Praticien hospitalier) , M. Klein (Professeur des Universités, praticien hospitalier)

Thyroid hormone directly affects the heart and the peripheral vascular system. It increases myocardial inotropy and heart rate and dilate peripheral arteries to increase cardiac output. Triiodothyronine (T3) enters the cardiac monocytes, and binds to nuclear T3 receptors. The complex then binds to thyroid hormone response elements of the genes for several cell constituents and regulates transcription of these genes, including those for Ca 2+ -ATPase and phospholamban in the sarcoplasmic reticulum, myosin, β-adrenergic receptors, adenylyl cyclase, guanine-nucleotide– binding proteins, Na+/Ca2+ exchanger, Na+/K+ – ATPase, and voltage-gated potassium channels. Non nuclear T3 actions on ion channels for sodium (Na+), potassium (K+), and calcium (Ca2+) ions represent an alternative way of action for thyroid hormone. Many electrocardiographic abnormalities have been described in hyperthyroidism including sinus tachycardia, atrial and ventricular extrasystoles, atrial fibrillation (AF), atrioventricular block and ventricular repolarisation abnormalities. AF is common in patients with hyperthyroidism, which predisposes to embolic events. Subclinical hyperthyroidism is associated with increased heart rate atrial arrhythmias, increased left ventricular mass impaired ventricular relaxation and reduced exercise performance. Overt hypothyroidism increases of coronary disease, pericardial effusion, systolic hypertension, myocardiopathy and congestive heart failure. Subclinical hypothyroidism is associated with impaired left ventricular diastolic dysfunction at rest, with systolic dysfunction in case of stress; the risk for atherosclerosis and myocardial infarction is increased.

甲状腺激素直接影响心脏和外周血管系统。它增加心肌收缩力和心率,并扩张外周动脉以增加心输出量。三碘甲状腺原氨酸(T3)进入心脏单核细胞,并与核T3受体结合。然后,该复合物与几种细胞成分的基因的甲状腺激素反应元件结合,并调节这些基因的转录,包括肌浆网中的Ca2+-ATP酶和磷蛋白、肌球蛋白、β-肾上腺素能受体、腺苷酸环化酶、鸟嘌呤核苷酸结合蛋白、Na+/Ca2+交换器、Na+/K+-ATP酶,以及电压门控钾通道。非核T3对钠(Na+)、钾(K+)和钙(Ca2+)离子通道的作用代表了甲状腺激素的另一种作用方式。甲状腺功能亢进症有许多心电图异常,包括窦性心动过速、心房和心室早搏、心房颤动(AF)、房室传导阻滞和心室再极化异常。AF在甲状腺功能亢进症患者中很常见,甲状腺功能亢进易发生栓塞事件。亚临床甲状腺功能亢进与心率增加、心律失常、左心室质量增加、心室舒张受损和运动能力下降有关。冠心病、心包积液、收缩性高血压、心肌病和充血性心力衰竭的甲状腺功能减退明显增加。亚临床甲状腺功能减退与静息时左心室舒张功能障碍受损有关,在压力情况下与收缩功能障碍有关;动脉粥样硬化和心肌梗死的风险增加。
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引用次数: 6
Communications interventriculaires 间通信
Pub Date : 2005-05-01 DOI: 10.1016/j.emcaa.2005.03.001
A. Chantepie (Professeur des Universités, praticien hospitalier)

Isolated ventricular septal defect (VSD) is the most common congenital cardiac malformation. VSD is also the most frequent defect associated to others congenital cardiovascular malformations. The numerous anatomic varieties of VSD explain the great variability of the clinical features and the differences in the natural history. With echocardiography and colour Doppler, the diagnosis of VSD is easier and more accurate than previously; so, pre-operative catheterization is no longer needed in most of the cases. The course of VSDs, depending on their size and location, may be early predicted in analysing their anatomic aspect by repeated echocardiography Doppler during the first months of life. Most of them become proportionally smaller with time and, finally, close spontaneously during infancy, childhood or adolescence. Few VSDs need early surgical closure to avoid complications of a large left-to-right shunt, such as cardiac failure and pulmonary hypertension secondary to a severe damage of pulmonary arteries. This management prevents Eisenmenger's syndrome, an irreversible situation corresponding to an inversion of the shunt in relation to high pulmonary vascular resistance. When VSD persists in adults, others complications such as infective endocarditis and aortic regurgitation may occur; therefore, strict surveillance and antibiotic prophylaxis are recommended in concerned patients. Owing to the improvement of cardiac surgery in low weight infants, the surgical closure of large VSD in infancy gives currently excellent results. Apical muscular VSD and multiple muscular VSD still present a particular surgical challenge: today, their closure may be achieved using a ventricular septal device during surgery or interventional catheterization. In this article, only isolated VSDs will be considered. Indeed, VSDs that constitute an integral part of a more complex malformation, such as the “tetralogie de Fallot” or complete atrio-ventricular defect, have a different presentation and require a specific treatment. Persistent VSD after correction of another cardiac malformation (aortic coarctation, transposition of great arteries, etc .) may be assimilated to isolated VSDs because they have usually similar clinical features and outcome.

孤立性室间隔缺损(VSD)是最常见的先天性心脏畸形。VSD也是与其他先天性心血管畸形相关的最常见的缺陷。VSD的众多解剖变异解释了临床特征的巨大可变性和自然史的差异。超声心动图和彩色多普勒对室间隔缺损的诊断比以前更容易、更准确;因此,在大多数情况下不再需要术前导管插入术。VSD的病程取决于其大小和位置,可以在生命的前几个月通过重复超声心动图多普勒分析其解剖方面进行早期预测。它们中的大多数会随着时间的推移而成比例地变小,最终在婴儿期、儿童期或青春期自发闭合。很少有VSD需要早期手术闭合,以避免大的左向右分流并发症,如肺动脉严重损伤继发的心力衰竭和肺动脉高压。这种治疗可以预防艾森曼格综合征,这是一种不可逆的情况,对应于与高肺血管阻力相关的分流倒置。当VSD在成人中持续存在时,可能会出现其他并发症,如感染性心内膜炎和主动脉瓣反流;因此,建议对相关患者进行严格的监测和抗生素预防。由于低体重婴儿心脏手术的改进,婴儿期大VSD的手术闭合目前取得了良好的效果。顶端肌性室间隔缺损和多发性肌性室隔缺损仍然是一个特殊的外科挑战:如今,它们的闭合可以在手术或介入导管插入术中使用室间隔装置实现。在本文中,只考虑孤立的VSD。事实上,构成更复杂畸形不可分割的一部分的室间隔缺损,如“法洛氏四联症”或完全性心房-心室缺陷,有不同的表现,需要特定的治疗。纠正另一种心脏畸形(主动脉缩窄、大动脉转位等)后的持续性室间隔缺损可能与孤立的室间隔缺损相似,因为它们通常具有相似的临床特征和结果。
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引用次数: 0
Chirurgie des cardiopathies congénitales à l’âge adulte 成人先天性心脏病的手术
Pub Date : 2005-05-01 DOI: 10.1016/J.EMCAA.2004.12.002
E. Belli, R. Roussin, C. Planché, A. Serraf
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引用次数: 2
Artériopathies iatrogènes et toxiques 医源性和毒性动脉疾病
Pub Date : 2005-05-01 DOI: 10.1016/J.EMCAA.2005.01.002
P. Cacoub, N. Limal, J. Piette
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引用次数: 2
Hémorrhéologie et cardiologie. Concept, physiopathologie, applications aux affections cardiologiques 血液和心脏病学。概念,病理生理学,在心脏病中的应用
Pub Date : 2005-05-01 DOI: 10.1016/J.EMCAA.2004.12.001
M. Boisseau
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引用次数: 0
Chirurgie des cardiopathies congénitales à l’âge adulte 成人先天性心脏病手术
Pub Date : 2005-05-01 DOI: 10.1016/j.emcaa.2004.12.002
E. Belli, R. Roussin, C. Planché, A. Serraf

The surgical management of congenital heart diseases in adult patients is characterized by the diversity of the anatomo-clinical situations and the difficulty of the specific surgical techniques. The pre-surgery evaluation is of great importance. The cardiac lesions encountered are often suitable for biventricular repair; however, there are also previously repaired hearts that require new surgical intervention due to the deterioration of the initial result. Encouraging results of surgery are observed, in terms of mortality and quality of life as well.

成人先天性心脏病的外科治疗具有临床情况多样性和特定手术技术难度大的特点。术前评估非常重要。遇到的心脏病变通常适合双心室修复;然而,由于最初结果的恶化,也有以前修复的心脏需要新的手术干预。手术在死亡率和生活质量方面也取得了令人鼓舞的结果。
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引用次数: 2
Traitement non pharmacologique de l'hypertension artérielle 高血压的非药物治疗
Pub Date : 2005-05-01 DOI: 10.1016/J.EMCAA.2005.03.002
J. Blacher, S. Czernichow, P. Iaria, J. Bureau, O. Roux, T. Kondo, B. Tournier, M. Cocaul, I. Moreau, J. Detienne, M. Safar
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引用次数: 4
Traitement non pharmacologique de l'hypertension artérielle 高血压的非药物治疗
Pub Date : 2005-05-01 DOI: 10.1016/j.emcaa.2005.03.002
J. Blacher, S. Czernichow, P. Iaria, J.-M. Bureau, O. Roux, T. Kondo, B. Tournier, M. Cocaul, I. Moreau, J.-P. Detienne, M. Safar

All guidelines on the management of arterial hypertension stress on the lifestyle measures have demonstrated their efficacy on blood pressure reduction. Although their effect on the cardiovascular risk remains hypothetical, they are, or should be, instituted in every hypertensive or pre-hypertensive subject. The five lifestyle measures that are widely agreed as lowering blood pressure are: 1) weight reduction, 2) reduction of excessive alcohol intake, 3) physical exercise, 4) reduction of salt intake and 5) augmentation of fruit and vegetable intakes and reduction of saturated and total fat intakes. It is reasonable to consider that these lifestyle modifications are, at best, additive to pharmacological treatment. As such modifications are difficult to institute and maintain in individuals, health education and public health campaigns should help practitioners in better controlling blood pressure in the general population.

所有关于动脉高压压力管理的生活方式措施指南都证明了其降压效果。尽管它们对心血管风险的影响仍然是假设的,但它们正在或应该在每个高血压或高血压前期受试者中实施。被广泛认为是降低血压的五种生活方式措施是:1)减肥,2)减少过量饮酒,3)体育锻炼,4)减少盐的摄入,5)增加水果和蔬菜的摄入,减少饱和脂肪和总脂肪的摄入。有理由认为,这些生活方式的改变充其量只是药物治疗的补充。由于这种修改很难在个人身上实施和维持,健康教育和公共卫生运动应该有助于从业者更好地控制普通人群的血压。
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引用次数: 6
期刊
EMC - Cardiologie-Angéiologie
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